Neuroendocrine Tumors

    Table of contents

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    Introdution

     

     

    Carcinoid

    • A tumor of (usually) GI origin that secretes various substances.
      •  As many as 40 substances identified: serotonin, histamine, tachykinin, kallikrein, prostaglandin
    • Origins:
      • Midgut Carcinoids (jejunum, ileum, cecum, appendix)
        • Rarely cause symptoms, as the liver metabolizes all vasoactive substances.
        • ONLY causes symptoms when metastasized to the liver
          • Flushing lasts 30s to 30min
          • Face, neck, upper chest become violaceous (purple) + mild burning sensation
          • Can become tachycardic during episodes or lightheaded
      • Foregut and hindgut (bronchial, gastric, appendiceal, rectal)
        • Produce symptoms without metastasis (direct circulation access)
        • Flushing red-brown hue, patchy, sharply demarcated, intensely pruritic
      • Bronchial
        • Flushing can last days, quite severe. 
    • Presentation:
      • Flushing and secretory diarrhea
        • Type of flushing depends on origin (see above)
        • Triggered by Alcohol, Chocolate, Beef
      • Also can have: 
        • Profuse sweating
        • Abdominal Pain
        • Bronchospasm
      • Longstanding:
        • Valvular heart disease (especially R-sided)
        • Facial telangiectasias
    • Diagnosis
      • Urine 24-hour collection for 5-hydroxyindoleacetic acid (5-HIAA)
      • Indium 111 pentetreotide scan --> determines if has somatostatin receptors
    • Management
      • Usually indolent (management with expectant observation, serial imaging), especially if well differentiated
        • Triple-phase contrast CT or MRI with gad.
      • Somatostatin analogues (octreotide or lanreotide) if:
        • Has somatostatin receptors
          AND
        • Symptomatic / shows growth
      • Anti-VEGF (sunitinib) and everolimus (mTOR agent) and oral cytotoxic combos (capecitabine and temozolomide)
      • BUT poor response to chemo agents 
      • If SEVERE symptoms:
        • hepatic arterial embolization, radiofrequency ablation, or surgical debulking, may be used to reduce symptomatic tumor bulk in the liver or to decrease hormone production.
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